Clinical manifestations of Ellis-van Creveld syndrome

Journal of the Indian Society of Pedodontics and Preventive Dentistry
C VinayR Chandra Sekhar

Abstract

Ellis-van Creveld syndrome (EVC) is a chondro-ectodermal dysplasia characterized by short ribs, polydactyly, growth retardation and ectodermal and heart defects. It is a rare disease complex and very few cases have been reported in dental literature. This condition is inherited as an autosomal recessive trait with variable expression. The present case report describes EVC in a 7-year-old girl, with all the tetrad of cardinal features. We found a rare dental aberration in form; appearance of single conical roots in primary molars. The management of children with EVC is multidisciplinary, with consideration for the high incidence of cardiac defects in these patients.

References

Jan 1, 1996·Ryōikibetsu shōkōgun shirīzu·S Ohdo
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Jun 6, 2007·Orphanet Journal of Rare Diseases·Geneviève Baujat, Martine Le Merrer

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Citations

May 3, 2011·Pediatric Cardiology·Christine B HillsJames H Moller
Jan 1, 2011·BMJ Case Reports·Karthik HegdePreeti P Nair
Oct 16, 2012·Journal of Pharmacy & Bioallied Sciences·Dhandabani JayarajNalliappan Ganapathy
May 26, 2012·Contemporary Clinical Dentistry·Ritesh Kalaskar, Ashita R Kalaskar
Jun 22, 2016·Journal of Cranio-maxillo-facial Surgery : Official Publication of the European Association for Cranio-Maxillo-Facial Surgery·Elif Bahar TunaBeyhan Tüysüz
Aug 26, 2014·BMJ Case Reports·SelvaKumar HaridossBharathan Rajendran
Jan 1, 2012·International Journal of Clinical Pediatric Dentistry·Subash SinghVijeev Vasudevan

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